Intrinsic path (PTT):

  • Exposed collagen + XII XI, IX X which activates thrombin to produce fibrin

Extrinsic path (PT):

  • Tissue factor + VII activated X fibrin


  • Is best single test to evaluate synthetic function of the liver

VII deficiency:

  • Causes long PT, normal PTT

Bleeding time:

  • Best pre-op test for patient on NSAIDS/ASA

X: is common to both pathways

XIII: Crosslinks fibrin (factor IIa) to form 'plug'

Vitamin K dependent Factors:

  • II, VII, IX, X, protein C and protein S

Factor XII:

  • Hagemann factor
  • Activated by cardiopulmonary bypass -> High need for heparin.

Factor V Leyden:

  • (A pro-coagulant) resistance to activated protein C
  • Most common congenital hypercoagulable disorder
  • Common cause of DVT
  • Found in 20% of pts w/ DVT) and 6% of US population.

V and VIII:

  • Are labile factors, low levels in stored blood.

Factor VIII & vWF:

  • Only factor not made in liver (made by reticuloendothelial system)(Endothelium)

Von Willebrand's Disease:

  • Long PTT, long bleeding time, (+) ristocetin test.
  • Most common congenital bleeding disorder
  • Only inherited coagulopathy with long bleeding time
  • vWF links the GpIb platelet receptor to the collagen







Autosomal DominantEdit

Mild bleedEdit

Reduce quantity of vWFEdit

Cryoprecipitate, DDAVP, Conjugated estrogensEdit


Autosomal DominantEdit

Mild bleedEdit

Reduce quality of vWFEdit



Autosomal RecessiveEdit

Severe bleedEdit

Reduce quantity of vWFEdit

Cryoprecipitate, DDAVP, Conjugated estrogensEdit


  • Causes release of vWF, useful also in patients on ASA or w/uremic platelets


  • Contains fibrinogen, vWF & VIII used in vWD, hemophilia A, and DIC if fibrinogen low.

Protein C: Degrades active V and VIII.

Protein S: Helps protein C

Acquired Antithrombin III deficiency:

  • Severe sepsis, trauma, burns, malignancies, extracorporeal circulation, and major surgery due to consumption coagulopathy
  • 70% of the normal concentration of ATIII represents risk to develop thrombosis.

Banked blood:

  • Is low in 2,3-DPG which increase Hgb affinity for O2 (left shift)

Glanzman's thrombasthenia:

  • Plts have IIb/IIIa receptor deficiency.
  • Decrease platelet aggregation

Bernard Soulier:

  • GpIb deficiency
  • Decrease adherence to exposed collagen

Hemophilia A:

  • VIII deficiency, sex-linked recessive
  • Replace to 100% levels pre-op
  • Have long PTT, normal PT
  • Epistaxis, IC bleeds and hematuria may occur
  • Tx: Factor VIII concentrate or cryoprecipitate
  • Newborn has VIII from mom, may not bleed at circumcision
  • Hemophiliacs joint: no aspiration, ice, range of motion therapy, give factor VIII.

Hemophilia B:

  • IX deficiency = Christmas disease, also sex-linked recessive
  • Achieve 50% levels pre-op
  • Tx: Factor IX concentrate or FFP

Lupus anticoagulant:

  • Antiphospholipid antibodies
  • Not necessarily with Lupus and generally pro-coagulant
  • Dx: long Russel viper venom time, long PTT, which does not correct by adding normal plasma.


  • Degrades fibrinogen
  • Is inhibited by alpha-2-plasmin inhibitor


  • Binds/activates ATIII and inactivates factors 9-10-11-12
  • Prolongs PTT
  • Counteract with protamine
  • Heparin resistance Antithrombin III deficiency give FFP as a source of antithrombin. Other alternative give commercially prepared antithrombin.


  • Cross reacts with NPH Insulin or in patients with previous protamine exposure
  • 4-5% of patients regardless protamine exposure gets reaction
  • Signs: Hypotension, Bradicardia, decrease heart function


  • (Epsilon Amino Caproic acid) inhibits fibrinolysis
  • Is the Tx for overdose of thrombolytics
  • Thrombin is best test to monitor thrombolysis


  • Low platelets, prolonged PT/PTT
  • Low fibrinogen, High fibrin split products


  • (White clot syndrome)
  • Thrombocytopenia due to anti-platelet antibody (IgG-PF4 AB) causing platelet aggregation/destruction/thrombosis
  • Generally see after 5 days of heparin, less frequent with LMWH
  • Tx: stop heparin, argatroban, hirudin, ancrod or dextran to anticoagulate.


  • PGI2: from endothelium
  • Decrease platelet aggregation, vasodilatation, bronchial relaxation


  • From platelets, opposite effects to PGI2

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